Myelofibrosis Cancer Symptoms, Stages, Prognosis, Treatment

Learn all about myelofibrosis signs and symptoms stages and treatments according to stages. It can affect people at any age, including children, but it’s most common in people over 50.

An important constant feature of MF is the overproduction of platelet-forming cells, called “megakaryocytes,” in the marrow. This causes too many platelets to be released into the blood and chemicals called “cytokines” to be released into the marrow. The cytokines stimulate the development of scar tissue in the marrow, called fibrosis. Paradoxically, the number of megakaryocytes can become so abnormal that platelet production decreases in some patients.

Severe anemia, easy bruising or bleeding, and Many patients will also experience intense itching, known as pruritis, caused by inflammation in the body. Multiple infections can result because of the lack of healthy blood cells. An enlarged spleen (splenomegaly) and/or liver (hepatomegaly) may also occur. When the bone marrow scars, the liver and spleen try to make blood cells to compensate (called extramedullary hematopoiesis), causing these organs to swell.

Approximately 25-50% of patients will have symptoms from an enlarged spleen at diagnosis, including pain with deep breaths, loss of appetite and feeling full after eating a small amount (called early satiety). Extramedullary hematopoiesis can also occur in other parts of the body (lymph nodes, spinal cord, and lungs), causing swelling in these areas, and leading to symptoms. Approximately 50-70% of patients report fatigue at the time of diagnosis. People may also develop chest pain or dizziness as the heart has to work harder to get adequate oxygen to the brain and other organs when the red blood cell count is low. A minority of patients report “constitutional symptoms”, which include weight loss, low-grade fever, and night sweats.

The diagnosis may be suspected from the results of a routine blood test called a full blood count. This test counts the number of red blood cells, white blood cells and platelets in the blood. About 90 percent of people with MF have a mutation (a change in their DNA) in one of three genes (JAK2, CALR or MPL). All three of these gene mutations cause abnormal signaling in the JAK pathway, which regulates blood cell production.

Myelofibrosis Cancer Symptoms, Stages, Prognosis, Treatment

What is Myelofibrosis Cancer?

Myelofibrosis is pronounced my-eh-lo-fy-bro-sis. It’s a rare blood disorder. Myelo means bone marrow and fibrosis relates to the development of fibrous or scar tissue. So myelofibrosis is a condition that causes scarring of the bone marrow. Myelofibrosis is a rare blood disorder. It is not cancer, but this condition can go on to develop into acute leukaemia in some people. Myelofibrosis is an uncommon type of chronic leukemia a cancer that affects the blood-forming tissues in the body. Myelofibrosis belongs to a group of diseases called myeloproliferative disorders.

Primary Myelofibrosis

Primary myelofibrosis (MF) is a chronic blood cancer in which excessive scar tissue forms in the bone marrow and impairs its ability to produce normal blood cells. Myelofibrosis primarily develops without a known cause (called primary myelofibrosis); however, other myeloproliferative diseases can progress into myelofibrosis.

Myelofibrosis Symptoms

Myelofibrosis symptoms and signs may include:

  • Tiredness, weakness, or shortness of breath with mild exertion
  • Fullness, discomfort or pain in the left upper area of the abdomen
  • Fever
  • Night sweats
  • Weight loss or malnutrition
  • Bone pain
  • Itching (pruritus)
  • Bruising
  • Susceptibility to infection
  • Abdominal distension/fluid retention (if portal hypertension or increased blood pressure within the portal vein is present)
  • Compromised liver function
  • Abnormal growth of blood forming cells outside of the bone marrow
  • Gout (inflammation in the joints)
  • Nosebleeds
  • Abnormal vaginal bleeding
  • Bleeding gums

Myelofibrosis Life expectancy and Prognosis

Life expectancy varies from person to person but it may be reduced. Your haematologist will be able to provide you with an accurate picture for your individual situation. Predicting the prognosis, or outcome, of MF is very difficult and depends on many factors.

  • Being over the age of 65
  • Experiencing symptoms that affect your entire body, such as fever, fatigue, and weight loss
  • Having anemia, or a low red blood cell count
  • Having an abnormally high white blood cell count
  • Circulating blood blasts greater than 1 percent

These factors are used in the IPSS, or international prognosis scoring system, to help doctors predict the average years of survival. People, who don’t meet any of the above problems, excluding age, are considered in the low risk category and have a median survival of over ten years. Meeting one of the criteria above reduces the survival to eight years, and meeting three or more can lower the expected survival to around two years.

Myelofibrosis Treatments

Myelofibrosis treatments include the following options;

  • Blood transfusion
  • Chemotherapy
  • Hydroxycarbamide is the most commonly used chemotherapy drug to treat MF.

Chemotherapy may affect fertility. People taking it are advised not to get pregnant or father a child, as there may be a risk of harming the developing baby. Contraception should be used while taking the drug and also for a few months afterwards. Other chemotherapies are Melphalan, Busulphan, Cladribine and Radioactive Phosphorous (P32).

  • Targeted (biological) therapies
  • Removing the spleen (splenectomy)
  • Radiotherapy
  • Stem cell transplant (from a donor)
  • Androgen therapy.

Other drug therapies include;

  • Thalidomide
  • JAK inhibitors, such as ruxolitinib (Jakavi), are a new type of treatment. They work by blocking (inhibiting) the faulty JAK2 gene
  • Interferon alpha
  • Danazol
  • Steroids
  • Erythropoietin

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